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Sinir Sistemi Cerrahisi Derg 1(3):190-194, 2008

Dumbbell-Shaped Neurofibroma of the


Upper Cervical Spine: A Case Report
Feyzi Birol SARICA, Kadir TUFAN, Melih EKNMEZ, Blent ERDOAN,
Orhan EN
Bakent University Medical Faculty, Department and Neurosurgery, Ankara

Spinal neurofibromas are the most prevalent group of spinal tumors. They occur sporadically
or in association with Neurofibromatosis type-1 (NF1, von Recklinghausen disease). A
neurofibromas developing a dumbbell tumor is a situation which is quite often seen. Surgical
intervention is indicated when myelopathy and motor deficits develop in the case of paraspinal
neurofibromas. The goal of surgery is total removal of the tumor. However, in selected cases partial removal of the tumor with adequate spinal cord decompression can be preferred to prevent
severe complications such as vertebral artery injury. We present a case of neurofibroma with neck
and shoulder pain and dumbbell tumor formation at the level of C1 that was in close relation with
the vertebral artery. Possible surgical interventions are discussed.
Key words: Dumbbell formation, spinal neurofibroma, surgical treatment, upper servical
area
J Nervous Sys Surgery 2008; 1(3):190-194

st Servikal Omurilik Blgesinde Gzlenen Kum Saati eklinde Nrofibroma: Olgu Sunumu
Spinal nrofibromlar spinal tmrlerin nemli bir ksmn oluturur. Sporadik yada
Nrofibromatozis tip-1 (NF1, von Recklinghausen hastal) ile birlikte grlr. Nrofibromann
Dummbell tmr eklinde geliim gstermesi olduka sk grlen bir durumdur. Paraspinal
nrofibroma olgularnda myelopati veya motor defisit gelitii durumlarda cerrahi endikasyon
doar. Cerrahide ama tmrn total karmdr. Ancak seilmi vakalarda, vertabral arter yaralanmas gibi ar komplikasyonlardan kanmak amacyla, yeterli spinal kord dekompresyonu
salayacak ekilde parsiyel karm seilebilir. Bu makalede, C1 dzeyinde vertabral arter ile
yakn ilikide olan, boyun ve omuz ars ile prezente olan dummbbell nrofibroma olgusu sunulmutur. Secilebilecek cerrahi giriim ekilleri tartlmtr.
Anahtar kelimeler: Cerrahi tedavi, kum saati formasyonu, spinal nrofibroma, st servikal
blge
J Nervous Sys Surgery 2008; 1(3):190-194

odular neurofibromas may occur anywhere on the peripheral nerves. They


frequently originate from the dorsal
roots and invade the sensorial branches. They
are capsulated and have a round and flexible
structure (3,5,7,8). No treatment is needed for
asymptomatic neurofibroma cases. Symptomatic
cases justify surgical treatment. Surgical treatment of neurofibromas is total removal. Posterior
laminectomy with unilateral facetectomy allows
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single-stage resection of dumbbell neurofibromas with significant intraspinal and paraspinal


components. Best results are obtained from patients with minimal neurological deficits in the
preoperative period (4,8,11,14).
CASE REPORT
A 61-year-female was admitted to the hospital
with pain over the neck, left shoulder and arm.

Sinir Sistemi Cerrahisi / Cilt 1 / Say 3, 2008

Dumbbell-Shaped Neurofibroma of the Upper Cervical Spine: A Case Report

Neurological examination revealed left-sided


spastic hemiparesis, more pronounced distally
hypoesthesia below C2 dermatome and hyperactive deep tendon reflexes. T1-weighted magnetic
resonance imaging (MRI) of the cervical spine
showed a hypointense mass measuring 1.5x2x2.5
cm and occupying the left neural foramen and
left half of the spinal canal. The lesion had a
prominent hyperintense center with a hypointense periphery on T2-weighted images. There was
a marked contrast enhancement of the periphery
of the lesion following gadolinium injection.
Intradural component of the mass was found to
compress the left side of the spinal cord while
the extradural component was close to the vertebral artery (Figures 1A and 1B). Vertebral
angiography was performed to determine the
course of the vertebral artery (VA) and to plan
the surgical procedure. Cranial diffusionperfusion MRI performed to depict cerebral
vascularization and collateral circulation was
normal.
The patient was operated via a posterior exposure with posterior arc of C1 and C2 removed.
During the partial resection, foraminal and extradural components were left in situ while the
intradural component of the tumor which compressed the spinal cord at the levels of C1 and C2
and contained nerve rootlets was totally excised
to achieve spinal cord decompression. Since the
case was considered as a neurofibroma, there
were possibilities of spinal cord compression by
the intradural component and adhesions of the
extradural component to the vertebral artery and
venous structures around it. Therefore, care was
taken not to cause traction during the dissection.
Histopathological diagnosis of the resected specimen was neurofibroma.
Postoperative neurological examination revealed left hemiparesis (4/5 motor strength).
Physical therapy and rehabilitation was given.
At 4 months followed-up, neurological examination was normal and neck pain was comple-

Sinir Sistemi Cerrahisi / Cilt 1 / Say 3, 2008

Figure 1. (A) A hyperintense mass overlying the left transverse


process was observed on preoperative, sagittal, contrastenhanced, T1-weighted magnetic resonance images of the
spine. (B) On axial, contrast-enhanced T1-weighted magnetic
resonance images of the patient performed preoperatively, a
hyperintense mass, with intra and-extradural components,
extending toward the left transverse process was observed.

tely resolved. Removal of intradural component


of the neurofibroma and adequate decompression of the spinal cord was seen on postoperative
cervical spine MRI (Figures 2A and 2B).

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F. B. Sarca, K. Tufan, M. ekinmez, B. Erdoan, O. en

extended laterally through the foramina. In these


dumbbell neurofibromas, the extraspinal part is
usually larger than the intraspinal part (14). Tumor
with may reach massive dimensions, be lobulated and exhibit cystic degeneration. Dumbbell
formation is important due to the attachment of,
especially, the extramedullary part to the surrounding tissues. Its vicinity to the VA is critical
(10,13,14)
.
Clinical findings develop as a result of local
compression of the ventral or motor nerve roots.
While root symptoms develop during the early
period, long-tract findings develop later. Cervical
and lumbar regions are more frequently invaded.
Radicular pain and disesthesia were present in
80 % cases. Motor weakness that we detected in
our case is seen in some 10 % of the cases
(5,8,11)
.

Figure 2. Sagittal (A) and axial (B) T2-weighted MR images of


the cervical spine of the patient taken 2 months after the operation clearly show that the intradural component of the
tumor at the level of C1-C2 was excised and there was no
spinal cord compression.

DISCUSSION
Of the paraspinal neurofibromas; 72 % were
with intradural extramedullar localization, where
as 14 % were with extradural, 13 % were with
dumbbell formation and 1 % was with intramedullary localizations (5,7,16). A neurofibroma in the
spinal canal, invading the peripheral segment of
the nerve by extending out of the intervertebral
foramen and presenting itself with a dumbbell
tumor is quite common. In the series of cases
presented by Seppala et al., 21 (66 %) of 32
neurofibromas demonstrated both intradural and
extradural tumor components, and 17 tumors
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Direct radiographs are sufficient to establish


diagnosis in 50 % of the cases. Pedicle erosion
and vertebral body scalloping are the most frequent findings on direct radiograph. Regular
expansion of the interpedicular distance and
intervertebral foramen may directly indicate the
presence of the dumbbell tumor. Thanks to its
sensitivity and specificity, MRI is quite important in detecting the disease, determining the
accompanying pathologies and following the
development of the complications in NF1 cases.
Neurofibromas appear as iso- or hyperintense to
the spinal cord on T1-weighted images while
they give hyperintense signal on T2-weighted
images. Dumbbell neurofibromas enhance regularly upon gadolinium administration (5,6,15).
No treatment is needed for asymptomatic neurofibroma cases. Symptomatic cases justify surgical treatment. Majority of the nerve fibers are
entrapped within tumoral tissue in dumbbell
neurofibroma cases, as in our case. It is impossible to remove the tumor without sacrificing the
nerve root and aggressive surgery may result in
severe neurological deficits (8,10). Thus, partial

Sinir Sistemi Cerrahisi / Cilt 1 / Say 3, 2008

Dumbbell-Shaped Neurofibroma of the Upper Cervical Spine: A Case Report

resection should be preferred in dumbbell neurofibroma cases that cause compression of the
spinal cord. As the aim of partial resection is to
resolve the symptoms, the extent of surgical
treatment is shaped according to the clinical
picture of the patient. Our patient presented with
myelopathic findings and, therefore, decompressive excision of the tumor was planned. In
decompression surgery, intradural component of
the tumor that compressed the spinal cord was
excised but foraminal and extradural components were left. Decompressive surgery is a
partial resection that carries with itself the risk
of recurrence and surgery may be needed
(5,8,9,10,11)
.
Dumbbell tumors with significant dissemination
into the paraspinal region may require complex
spinal exposure. Although two-stage operations
may be performed to manage the intraspinal and
paraspinal components separately, a single-stage
procedure is preferable. For cervical tumors, the
VA is another issue to be considered. In most
instances, meningiomas and nerve sheath tumors
receive little blood from the spinal cord and are
attached by few adhesions to the spinal cord.
Most cervical dumbbell tumors can be adequately accessed through a standard laminectomy
and complete unilateral facetectomy. As in our
case, this allows paraspinal access up to 3 cm
from the lateral dural margin. A second-stage
anterior procedure may be required if further
tumor extension is present (2,4,10,12).
The VA is consistently displaced anteromedially
by dumbbell neurofibromas of the cervical
spine. The artery is neither encased nor invaded
by these tumors but is separated from the tumor
capsule by a thin layer of periosteum and perivertebral veins. These tissues serve as an effective and easily developed plane of dissection
that is rarely associated with VA injury. Thus,
because of the low risk of either VA injury or its
potential ischemic consequences, preoperative
angiography and/or test occlusion or early intra-

Sinir Sistemi Cerrahisi / Cilt 1 / Say 3, 2008

operative control and mobilization do not seem


warranted (4,10,13).
The incidence of cervical spine instability after
unilateral facetectomy and varying degrees of
laminectomy is unknown. In an experimental
study by Cusick et al., isolated unilateral cervical facetectomy resulted in an average loss of
strength of 31.6 % in response to a constant flexion/compression load, as compared with an
intact motion segment (1). Although acute spinal
instability did not occur in the clinical study by
McCormick et al., the significant loss of mechanical integrity associated with unilateral facetectomy presented a continued risk of delayed instability from repetitive loading (11). This risk
probably increases in proportion to the amount
of concomitant laminar ant ligamentous disruption. Independent factors, such as patient age,
spinal mobility, individualized loading patterns,
and spinal level, might also be relevant.
Contralateral facet fusion prevents delayed instability.
Prognosis is excellent after the surgical resection. While pain is diminished in 80 % of the
cases, total remission occurs in 60 % of the
cases. Recurrence is very rare subsequent to
total excision. Recurrence after 3 years was
noted in one of 66 paraspinal neurofibroma patients who were treated by Levy et al. (8). However,
upper cervical neurofibroma cases characterized
by dumbbell formation, as in our case, are treated by partial resection, thus they have the risk
of recurrence. It is crucial to screen cervical
spine in these patients by advanced imaging
modalities such as MRI to detect recurrence
(5,10,11,14)
.
CONCLUSION
The most significant feature of dumbbell neurofibromas is the adhesion of the tumor to the
environment by enlarging the foramen and projecting outward from the spinal canal (8,10,11). The
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F. B. Sarca, K. Tufan, M. ekinmez, B. Erdoan, O. en

goal of surgery is total removal of the tumor.


Although a variety of surgical approaches for
these lesions is available, most cervical spine
dumbbell tumors can be effectively managed
with a single-stage posterior exposure with partial laminectomy and unilateral facetectomy
(4,8,10,13,14)
. However, in selected cases partial
removal of the tumor with adequate spinal cord
decompression can be preferred to prevent vertebral artery injury.

7.
8.
9.
10.
11.

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Sinir Sistemi Cerrahisi / Cilt 1 / Say 3, 2008

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